RESUMEN
We report a case of congenital accessory scrotum and pseudodiphallia with perineal lipoma. A 7-month-old boy was admitted to Xiamen Children's Hospital for a postnatal perineal mass. Physical examination found a mass of 4 cm×4 cm between the scrotum and anterior edge of the anus, with pigmented and wrinkled skin resembling the scrotum without a testicle. There was also a penis-like swelling on the surface of the mass without a urethral orifice. The penis and scrotum with bilateral testis were normal, as were the other organs. The patient's chromosome was 46,XY. Perineal ultrasound, MRI, and voiding cystourethrogram indicated no association between the mass and the penis, urethra, rectum, and anus. A resection of the mass and penis-like swelling and perineoplasty was performed because the normal penis was found to rotate clockwise about 90 degrees during the operation. The pathology showed that the masses were accessory scrotum and lipoma and the penile-like swelling was a pseudodiphallia without corpus cavernosum. Congenital accessory scrotum and pseudodiphallia with a perineal lipoma are rare. It is essential to clarify the anatomical relationship between the mass and genitourinary tract or anorectum before operation.